Is Reye's Syndrome Deadly?

Reviewed By: Pramod Kerkar, MD, FFARCSI

Is Reye's Syndrome Deadly?

Reye’s is life-threatening syndrome and extremely deadly. Yes, Reye’s is life-threatening syndrome and extremely deadly one. Though it is a rare disorder, if it left without timely treatment at the initial stages it may develop to severe illness, sometimes leads to death in children and adolescents. As this syndrome is biphasic, first phase infection is not at all fatal. Nonetheless, secondary phase involves serious of consequences such as liver impairment and brain dysfunction. If these symptoms not reduced on time it can progress to permanent damage resulting seizure, coma, and death. During the 70s to 90s, the mortality rate was higher i.e., more than 42% worldwide.

Pathophysiology of Reye’s

The cause of the Reye’s is still unknown and unclear. The pathophysiology of the disease starts with mitochondrial injury and disrupts the Krebs cycle (fatty acid β-oxidation and oxidative phosphorylation) in the human body. As a result, the high concentration of ammonia found in the liver induces brain inflammation i.e., most deadly encephalopathy condition. The pathophysiology of the Reye’s was similar to aspirin toxicity (Salicylate drugs). However, no strong report was presented on Reye’s syndrome related to aspirin intake. A lot of debate still continues by the expert related to cause of the disease.

Infectious Agent in Reye’s Syndrome and Reye-like Syndrome

A number of infectious agents have been associated with Reye’s syndrome and Reye-like syndrome. Initial onset of fever caused by several virus like influenza, varicella, parainfluenza, measles, adenoviruses, coxsackieviruses, cytomegalovirus, Epstein–Barr virus, HIV, hepatitis A and B, and rotavirus. Some bacterial agents involved in the cause of disease like Mycoplasma, Chlamydia, Shigella, and Salmonella.

Immune Response to Infectious Agent

Infectious viral agent cause an adverse effect on kupffer cells and inhibits the phagocytic mechanism. In patients serum and CSF, endotoxin factor is reported which cause the release of tumor necrosis factor (TNF). This factor in the human body cause severe inflammation and lead to several immune system diseases.

Five Stages of Reye’s

Reye syndrome can be described in five stages.

Stage 1: It is the initial phase generally starts with rash and headaches with no increase in temperature (Fever). Tiredness, vomiting, confusion, and hallucination are some of the symptoms observed from mild to moderate level.

Stage 2: Overactive and irrational behavior, hyperventilation, fatty liver, and stuporous state are witnessed in the patients.

Stages 1 to 3 are considered as mild to moderate disease and stages 4 and 5 is the most life-threatening illness stages of Reye's syndrome. In some cases, after recovery from Reye’s, certain long-lasting symptoms identified in patients such as hearing problem, poor vision, memory and swallowing difficulties.

No Suitable Therapy for Reye’s

There is no appropriate treatment for Reye’s and physicians treat the patients with respect to their symptoms and laboratory results. Hence, there is a lot of risk and death report case associated with Reye’s syndrome. Early treatment to reduce the symptoms is essential. Low glucose level and abnormal intracranial pressure are the most diagnosed symptoms in Reye’s. Administration of 10% glucose solution every 24 hours and sustaining the intracranial pressure is a necessary treatment for Reye’s patients.

Cerebral edema is managed by fluid restriction, diuresis, and corticosteroids. Pentobarbital prescribed to patients to reduce the brain metabolic stresses. Adequate oxygen or O2 supply is required for controlling pressure by means of intubation could save the patient from death. the Antiepilepsy drugs for seizure and treatment for removal of the accumulation of ammonia are necessary to stop the progress of the syndrome to more severe form.